Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis.

OBJECTIVE: To describe a novel, sonographic approach for in-utero evaluation of normal and abnormal aortic arch. METHODS: Aortic arch was evaluated by imaging of the axial view of the upper fetal mediastinum. The normal left aortic arch was defined by the V-shaped appearance of the junction between the ductus arteriosus and aortic arch, with the trachea situated posteriorly. Right and double aortic arches were diagnosed when the great vessels appeared U-shaped, with intermediate location of the trachea. RESULTS: Between 1997 and 1999, 18 347 women were scanned in three prenatal centers, and pathological findings were prospectively recorded. In a retrospective analysis of the records, we identified 19 fetuses (0.1%) with atypical, U-shaped appearance, and no other structural abnormalities present. With the exception of one fetus with a ventricular septal defect, no congenital cardiac defects were present. Right aortic arch was found in 18 cases, while color Doppler made it possible to diagnose one case with double aortic arch, and one fetus was demonstrated as having Kommerell's diverticulum. In all 18 cases, a left descending aorta and left ductus arteriosus were present, the latter coursing to the left of the trachea, forming a loose partial vascular ring. All were asymptomatic at birth and early infancy. The fetus with double aortic arch that had a true vascular ring underwent early infantile correction. CONCLUSIONS: It is possible to diagnose right and double fetal aortic arch using prenatal ultrasound. The use of color Doppler facilitated in-utero evaluation of possible complications, such as true vascular ring.     

Prenatal Diagnosis of Vascular Rings

Objectives. The purpose of this series was to identify vascular rings prenatally on fetal echocardiograms. Methods. We reviewed the fetal echocardiograms and clinical histories of 9 patients with a diagnosis of vascular rings at our institution from 2004 to 2009. Eight patients had a prenatal diagnosis by fetal echocardiography. One other patient who had undergone fetal echocardiography had a diagnosis of a vascular ring and a cervical arch only postnatally. Results. Among the 8 patients with a prenatal diagnosis (4 with a double aortic arch and 4 with a right aortic arch [RAA], an aberrant left subclavian artery, and a left ductus arteriosus [LDA]), the vascular ring was isolated in 4 and associated with other structural congenital heart disease in 4. In all 8 patients with a prenatal diagnosis, the vascular ring was identified by cephalad transducer sweeps from a 3‐vessel view (3VV) with and without color Doppler imaging, which revealed vascular structures coursing around the trachea. Seven of these patients had postnatal confirmation of the abnormality (there was 1 pregnancy termination without autopsy). The only known vascular ring missed at fetal echocardiography but diagnosed after birth was that associated with a cervical RAA (which could not be visualized in cross‐sectional sweeps) and an LDA evaluated only late in pregnancy. Only 3 of the 8 postnatally treated neonates had clinical symptoms of the vascular ring, and 5 underwent surgical division of the ring. Conclusions. Vascular rings can be largely identified before birth with cephalad sweeps from the 3VV with attention to the relationship of the aortic and ductal arches to the trachea on fetal echocardiograms.     

胎儿右位主动脉弓的产前超声诊断及分析

目的提高胎儿右位主动脉弓的产前超声诊断正确率。方法分析总结我院诊断的51例右位主动脉弓胎儿产前超声特征。结果 51例右位主动脉弓胎儿中,右位主动脉弓并左锁骨下动脉迷走30例(其中右位主动脉弓并左无名动脉迷走1例),镜面右位主动脉弓21例。单纯右位主动脉弓24例,合并复杂心内外结构畸形24例(镜面右位主动脉弓19例),形成血管环29例。结论不同类型的胎儿右位主动脉弓有不同的结构特点,并有其特殊的产前超声表现,部分镜面右弓和右位主动脉弓并动脉迷走时在产前诊断仍有一定的难度。     

应用超声三血管气管切面诊断胎儿主动脉弓异常

  目的  研究超声三血管气管切面对于胎儿主动脉弓异常的诊断价值。  方法  回顾性分析2010年5月至2014年5月北京协和医院诊断的17例胎儿主动脉弓异常的产前超声图像资料, 并与产后超声及病理结果进行对照, 分析不同类型主动脉弓异常的产前超声特征, 重点关注三血管气管切面的表现。  结果  17例主动脉弓异常在三血管气管切面均有阳性表现。其中主动脉弓缩窄3例, 表现为主动脉弓细小; 主动脉弓离断7例, 表现为主动脉变细, 不能连接至降主动脉; 右位主动脉弓合并迷走左锁骨下动脉5例, 可见环绕气管的"U"形血管环; 右位主动脉弓合并镜像分支2例, 可见主动脉弓位于气管右侧, 动脉导管不汇入降主动脉。  结论  三血管气管切面是提示胎儿主动脉弓异常敏感且有效的切面, 中孕超声筛查时应重视该切面, 早期发现异常并给予适当的遗传咨询。     

右位主动脉弓胎儿超声心动图诊断分析

目的总结右位主动脉弓胎儿超声心动图表现及其血管特征.方法对26例超声心动图诊断为右位主动脉弓胎儿的超声心动图表现进行总结分析并观察其临床结局.结果26例右位主动脉弓胎儿中12例为孤立右位主动脉弓,2例为双主动脉弓,2例合并永存左上腔静脉,7例合并法洛四联症,3例合并永存动脉干.26例右位主动脉弓胎儿超声心动图均在上纵隔三血管气管观作出诊断.其中21例为右位主动脉弓合并迷走左锁骨下动脉、左位动脉导管,形成围绕气管食管的“U”字形血管环;2例双主动脉弓形成围绕气管食管的“O”字形血管环;3例无上述典型超声心动图图像特征.26例中12例活产儿经产后超声心动图或X线检查证实为右位主动脉弓;2例双主动脉弓经外院胎儿磁共振检查证实为右位主动脉弓;10例合并复杂先天性心脏病的胎儿均引产(6例尸体解剖检查证实为右位主动脉弓,4例未行尸体解剖检查);2例失访.结论胎儿右位主动脉弓有特征性超声表现,上纵隔三血管气管观可作为诊断胎儿右位主动脉弓的主要切面观;右位主动脉弓可孤立存在或同时合并其他心脏畸形.     

超声断层显像技术诊断胎儿主动脉弓畸形

目的 探讨时空影像关联（STIC）技术结合超声断层显像（TUI）诊断胎儿主动脉弓异常的价值。方法 回顾性分析45胎主动脉弓畸形胎儿的产前超声资料。以二维超声心动图（2DE）于三血管-气管切面判断主动脉弓相对于气管的位置,寻找异常头臂动脉。以STIC技术采集容积数据后,分别在横断面及冠状面行TUI重建,于横断面TUI切面判断弓的位置;于冠状面TUI切面寻找迷走左、右锁骨下动脉及双侧主动脉弓汇合处。结果 45胎中,5胎双主动脉弓,9胎左位主动脉弓合并右锁骨下动脉迷走,31胎右位主动脉弓。STIC-TUI法及2DE对胎儿主动脉弓畸形的诊断率分别为97.78%（44/45）及71.11%（32/45）,差异有统计学意义（P<0.01）。结论 STIC-TUI法可准确诊断胎儿主动脉弓畸形;标准化容积数据后处理方法使临床推广成为可能。     

胎儿孤立完全性血管环常见类型产前超声诊断研究

目的通过胎儿超声心动图观察孤立完全性血管环（CVR）的特征,测量气管横截面积（TA）和血管环面积（VRA）,以期为围产期管理提供依据。 方法选取2010年8月至2015年11月首都医科大学附属北京安贞医院超声心动图二部胎儿心脏病母胎医学会诊中心胎儿数据库资料中诊断孤立完全性血管环胎儿92例作为病例组,并选取同期正常胎儿92例作为对照组,于三血管气管观观察超声特征并测量主动脉弓水平气管横截面积（TA）和血管环面积（VRA）。 结果病例组92例孤立完全性血管环胎儿气管横截面积测值（2.39±1.26）mm²小于对照组正常胎儿（5.27±1.49）mm²测值,病例组92例孤立完全性血管环胎儿血管环面积测值为（48.92±19.10）mm²,右位主动脉弓伴左位动脉导管胎儿77例,表现为"U"字形血管环,双主动脉弓胎儿15例,表现为"O"字形或"λ"形血管环。 结论胎儿超声心动图能诊断孤立完全性血管环,孤立完全性血管环对胎儿气管有一定程度受压,气管横截面积测值小于对照组正常胎儿。     

胎儿完全性血管环的产前超声诊断研究

目的 探讨产前超声诊断胎儿完全性血管环的临床价值。方法 回顾性分析6例经引产后尸检证实的完全性血管环胎儿的产前超声表现,总结其声像图特点。结果 5例为右位主动脉弓伴左侧动脉导管构成的完整血管环;1例为双主动脉弓构成的完整血管环。在三血管-气管平面显示5例主动脉弓走行在气管右侧,与左侧动脉导管在气管后方交汇,构成“U”形动脉弓,包绕气管;1例主动脉弓在气管前呈“Y”形分支走行在气管两侧并在气管后方汇合,构成“O”形动脉环,包绕气管。结论 产前超声诊断胎儿双主动脉弓及右位主动脉弓伴左侧动脉导管构成的完全性血管环具有重要临床价值。三血管-气管平面是产前超声诊断胎儿血管环的重要切面。     

Prenatal detection of ductal-dependent congenital heart disease: how can things be made easier?

OBJECTIVE: To improve the detection of ductal dependence in fetuses with severe anomalies of the outflow tracts by observing, with directional power Doppler, reverse flow through the aortic arch or ductus arteriosus in a transverse view of the upper mediastinum. METHODS: A slight cranial move of the ultrasound beam from the three-vessel view allows the transverse view of the aortic arch and ductus arteriosus to be visualized simultaneously. This view is orthogonal to the fetal body axis and parallel to the plane of the four-chamber view. In normal fetuses, directional power Doppler interrogation at this level identifies forward flow in both oblique vessels. RESULTS: We examined 43 fetuses with cardiac defects. In five of the cases, there was reversed flow in the aortic arch or ductus arteriosus in addition to severe anomalies of the outflow tracts, including four with hypoplastic left ventricle and one with pulmonary atresia. CONCLUSIONS: Prenatal detection of reversed flow in the aortic arch or ductus arteriosus is associated with complex congenital heart disease with major diminution of forward flow to the corresponding great vessels.     

基层医院产前胎儿心脏超声筛查漏诊情况分析

目的探讨深圳地区基层医院产前胎儿心脏超声筛查常见漏诊疾病及原因。方法对我院在基层医院初诊中漏诊胎儿心脏畸形的会诊病例103例以四腔心、左右心室流出道和三血管气管切面为基本筛查切面进行产前胎儿心脏超声筛查,分析深圳地区基层医院胎儿心脏畸形的常见漏诊情况。结果本组四腔心切面遗漏左位上腔静脉33例,完全性肺静脉异位回流11例;左右心室流出道切面漏诊12例法洛四联征,6例永存动脉干,5例右室双出口,5例肺动脉瓣闭锁;三血管气管切面漏诊迷走锁骨下动脉20例,主动脉弓离断7例,主动脉弓缩窄4例。结论各个筛查标准切面细节观察不足是深圳地区基层医院漏诊胎儿心脏畸形的主要原因。     

Role of the 3‐Vessel and Trachea View in Antenatal Detection of Tetralogy of Fallot

Prenatal diagnosis of tetralogy of Fallot remains less frequent compared to other major congenital heart defects. In this study, we examined how often the 3‐vessel and trachea view was abnormal in a large series of prenatally diagnosed cases of tetralogy of Fallot. In addition, we compared its sensitivity to that of the traditional outflow tract views for detection of tetralogy of Fallot. We found that both views were abnormal in all fetuses with tetralogy of Fallot, showing reversed aortic‐to‐pulmonary valve and aortic arch isthmus‐to‐ductus arteriosus ratios in the outflow tract and 3‐vessel and trachea views, respectively. However, as a single measured marker, the enlarged aortic arch isthmus on the 3‐vessel and trachea view appears to be the most sensitive for tetralogy of Fallot.     

二维超声结合时空关联成像诊断胎儿先天性血管环

目的 探讨二维超声结合时空关联成像(STIC)诊断胎儿先天性血管环的价值。方法 对于我院接受胎儿超声心动图、可疑或明确先天性血管环的胎儿进行STIC数据采集,对产前超声诊断结果与产后新生儿超声心动图、CT及引产后胎儿尸体病理解剖结果进行对比分析。结果 二维超声心动图结合STIC共检出胎儿先天性血管环83胎,其中双主动脉弓5胎,右位主动脉弓、左位动脉导管并迷走左锁骨下动脉59胎,左位主动脉弓、左位动脉导管并迷走右锁骨下动脉19胎,漏诊1胎肺动脉吊带。84胎先天性血管环胎儿中,69胎经产后新生儿超声心动图、CT证实,2胎经尸体解剖证实,13胎失访。结论 超声心动图可用于诊断胎儿先天性血管环,STIC技术可更精确、直观地显示先天性血管环的空间位置关系。     

产前超声三血管多切面和主动脉冠状及矢状切面诊断胎儿先天性血管环

目的 观察产前超声三血管多切面和主动脉冠状及矢状切面诊断胎儿先天性血管环的价值。方法 回顾性分析42胎经产前超声诊断的先天性血管环胎儿,于超声三血管多切面、主动脉冠状及矢状切面观察上腔静脉、主动脉弓、肺动脉及动脉导管与气管的位置关系等,并结合产后随访结果分析各切面用于诊断胎儿先天性血管环的价值。结果 42胎中,19胎为右位主动脉弓,其中16胎为右位主动脉弓+迷走左锁骨下动脉+左侧动脉导管,3胎为右位主动脉弓伴镜像分支+左侧动脉导管;17胎为左位主动脉弓伴迷走右锁骨下动脉;3胎为双主动脉弓,其中2胎为右弓优势型、1胎为均衡型;3胎为肺动脉吊带。15胎合并心内畸形。8名孕妇因胎儿染色体异常终止妊娠,9名孕妇于外院分娩而失访;25例新生儿超声心动图或CTA所见均与产前检查结果相符。结论 产前超声三血管多切面结合主动脉冠状及矢状切面有助于检出胎儿先天性血管环。     

Fetal sonographic diagnosis of aortic arch anomalies.

Aortic arch anomalies refer to congenital abnormalities of the position or branching pattern, or both of the aortic arch. Although aortic arch anomalies are not uncommon, reports on their prenatal diagnosis are scarce. Insight into the hypothetical arch model is crucial to understanding anomalies of the aortic arch in the fetus. Recognition of the trachea, three major vessels, ductus arteriosus and descending aorta in the axial views of the upper mediastinum is necessary for a complete fetal cardiac assessment. Clues to aortic arch anomalies include abnormal position of the descending aorta, absence of the normal 'V'-shaped confluence of the ductal and aortic arches, a gap between the ascending aorta and main pulmonary artery in the three-vessel view, and an abnormal vessel behind the trachea with or without a vascular loop or ring around the trachea. Meticulous attention to anatomic landmarks will lead to successful prenatal diagnosis of important vascular rings making early postnatal management possible.     

Color Doppler examination of the outflow tracts of the fetal heart: a technique for identification of cardiovascular malformations.

The objective was to describe a technique using color Doppler to identify the outflow tracts of the fetal heart by directing the ultrasound transversely through the fetal chest. One hundred second- and third-trimester control fetuses were examined with real-time and color Doppler ultrasound. The ultrasound beam was directed cephalad, in the same transverse plane used to image the four-chamber view, and the outflow tracts were examined. Four fetuses with abnormal cardiovascular anatomy were examined using the above approach, to study the anatomical relationships of the outflow tracts identified with color Doppler ultrasound in normal fetuses.When the ultrasound beam was directed immediately cephalad to the four-chamber view, the aorta was identified as it exited the left ventricle. Further movement of the ultrasound beam cephalad identified the following vessels in a single plane: the main pulmonary artery perpendicular to the ascending aorta; the left pulmonary artery branching from the main pulmonary artery; the full length of the ductus arteriosus; and the transverse arch of the aorta. The ascending aorta, main pulmonary artery, ductus arteriosus and transverse aortic arch were identified in 100% of fetuses. Four fetuses with abnormalities of the outflow tracts (aortic stenosis, aortic regurgitation, pulmonary stenosis and premature constriction of the ductus arteriosus) were imaged using this approach in which pathology was readily identified.This technique enables rapid identification of the outflow tracts in second- and third-trimester fetuses using color Doppler and accurately identifies abnormalities of these vessels.     

Subclavian and pulmonary artery steal phenomenon in a patient with isolated left subclavian artery and right aortic arch

We describe a patient with an isolated left subclavian artery associated with right aortic arch, patent ductus arteriosus, and ventricular septal defect. As the isolated left subclavian artery is supplied by the left vertebral artery in which blood flows in the retrograde direction, this anomaly is usually responsible for a congenital subclavian steal phenomenon. Atrophy of the left cerebral hemisphere and inverted left vertebral arterial flow were clearly depicted by echoencephalography in this patient, whose subclavian artery was connected to the main pulmonary artery by a patent ductus arteriosus. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2013.     

胎儿右位主动脉弓产前超声诊断、妊娠结局及漏误诊分析

目的探讨并总结胎儿右位主动脉弓（RAA）超声图像特征、鉴别诊断方法,分析其漏误诊原因,提高胎儿RAA产前超声诊断准确率。 方法选取2014年1月至2017年12月黑龙江省哈尔滨市红十字中心医院31例经产后小儿超声心动图检查、手术或引产后尸体解剖病理证实的胎儿RAA病例,总结胎儿RAA及其合并圆锥动脉干畸形产前超声图像特征、鉴别诊断方法,分析其漏误诊原因并随访其妊娠结局。应用胎儿心脏标准超声切面的灰阶和彩色多普勒血流成像,进行胎儿心脏系统超声检查。当三血管气管切面（3VT）发现主动脉弓位于气管右侧时,再获取气管及其分支冠状切面进一步明确主动脉弓、动脉导管弓与气管的位置关系。 结果产前超声诊断41例胎儿RAA,活产20例,引产15例,失访6例;产后证实31例,产前超声正确诊断25例（25/31,80.65%）,漏误诊6例（6/31,19.35%）。2例左位主动脉弓产前超声均未获取胎儿气管及其分支冠状切面,在3VT切面将支气管横断面误认为气管横断面而误诊为RAA,2例RAA伴左锁骨下动脉迷走（ALSA）左位动脉导管（LDA）,将左颈总动脉误认为发育不良的左弓而误诊为双主动脉弓（DAA）,2例RAA合并心脏圆锥动脉干畸形产前超声漏诊RAA。31例胎儿RAA在3VT切面显示主动脉弓位于气管右侧,不同类型的胎儿RAA在3VT切面形成血管环或无血管环。胎儿RAA合并圆锥动脉干畸形在3VT切面和气管及其分支冠状切面有不同超声表现。20例活产胎儿中13例孤立性RAA、2例合并永存左上腔、1例合并2~3腰椎椎体融合、2例合并室间隔缺损手术治疗、最大年龄随访至3岁半,无呼吸道消化道压迫症状,小儿状态良好。1例IDD型矫正型大动脉转位并RAA,未经手术治疗,目前小儿11个月,状态良好。1例合并食道闭锁产后14 d新生儿死亡。6例行胎儿染色体核型检查,5例染色体核型正常,1例合并心内外严重多发畸形胎儿染色体核型检查为18-三体。 结论3VT切面是诊断胎儿RAA的主要切面,联合应用气管及其分支冠状切面可提高产前超声诊断准确率。单纯性RAA多数预后好、RAA合并严重心内外畸形预后与其合并畸形严重程度有关。     

胎儿双侧锁骨下动脉切面在迷走右锁骨下动脉诊断中的应用价值

目的探究胎儿双侧锁骨下动脉切面在迷走右锁骨下动脉(ARSA)诊断中的应用价值。方法选择2018年4月至2019年4月入院接受胎儿心脏超声检查的978例孕妇为研究对象,采用彩色多普勒超声诊断仪观察胎儿三血管气管切面(3-VT)和双侧锁骨下动脉切面。统计孕妇产前超声检查结果中的ARSA检出情况。结果978例胎儿中3-VT切面异常者20例,包括6例ARSA、2例主动脉弓缩窄、2例法洛氏四联症、2例永存动脉干、1例右心发育不良、1例单心室、6例永存左上腔。双侧锁骨下动脉切面ARSA检出率为0.72%(7/978),其中6例与3-VT切面诊断结果一致,1例被3-VT切面扫查漏检。结论双侧锁骨下动脉切面可清晰显示左、右锁骨下动脉及其与左、右无名静脉、气管的位置关系,有助于提高产前ARSA检出率,预防严重畸形儿出生。     

超声心动图诊断胎儿双主动脉弓的价值

目的探讨超声心动图诊断胎儿双主动脉弓的临床价值。方法 8例双主动脉弓胎儿,超声对各切面超声特点进行分析。结果超声心动图诊断胎儿双主动脉弓6例,5例为右弓稍大于左弓,1例为左右弓内径基本相同。1例胎儿同时合并其他心内畸形,核型分析为21-三体。左、右侧主动脉弓上分别发出两支头臂动脉。超声心动图漏诊2例,为左弓内径明显小于右弓。结论胎儿双主动脉弓在三血管-气管切面显示,由左弓和右弓包绕气管形成的完全血管环,左弓和右弓上均仅见两支头臂动脉发出。超声心动图对胎儿双主动脉弓早期检出具有重要的临床价值。     

胎儿双主动脉弓的产前超声心动图诊断

目的探讨双主动脉弓胎儿产前超声心动图特征及产前诊断临床意义。方法对南京医科大学附属苏州医院2012年1月至2013年2月产前超声心动图检出的5例双主动脉弓胎儿的超声心动图特征、分型及临床结局进行总结分析。结果5例双主动脉弓胎儿超声心动图表现：（1）三血管气管观中主动脉与动脉导管失去正常“V”字形结构,升主动脉发出左、右主动脉弓围绕气管,彩色多普勒血流成像示环状血流信号围绕气管。（2）超声心动图示5例胎儿中3例为右主动脉弓优势型,2例为左、右主动脉弓平衡型。（3）1例（例2）胎儿合并膜部室间隔缺损、永存左上腔静脉：1例（例4）胎儿合并半椎体畸形,3例胎儿未发现合并其他畸形。5例胎儿均行磁共振检查并随访至引产或产后3个月,随访检查均证实胎儿为双主动脉弓畸形。结论双主动脉弓是胎儿严重的先天性心脏病,超声心动图是产前首选的诊断方法,三血管气管观是诊断双主动脉弓的有效切面。